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1/4. Hereditary cerebral cavernous angiomas: presentation as idiopathic familial epilepsy.

    In cavernous angiomas of the brain, abnormally dilated blood vessels are tightly clustered together with no intervening neural parenchyma. Commonly recognised as a sporadic finding, these lesions may also be inherited in an autosomal dominant fashion. This disorder was recognised as a common entity with the advent of MRI. As lesions were often not detected on CT imaging, symptomatic patients presenting with seizures were mistakenly diagnosed with an idiopathic seizure disorder. seizures are the commonest presenting symptom followed by haemorrhage and focal neurologic deficit. We present another cause of familial epilepsy that also emphasises the importance of MRI as the modality of choice in investigating seizures in children.
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2/4. Congenital cavernous angioma exhibits a progressive decrease in size after birth.

    CASE REPORT: We report a case of congenital intracranial cavernous angioma, which was initially found at a gestational age of 34 weeks in utero as a mass lesion associated with hydrocephalus. After birth, the patient was treated for hydrocephalus first by external drainage and then by ventricular peritoneal shunt. The natural course of the mass lesion was observed until the age of 8 months when the histopathological diagnosis confirmed the cavernous angioma after tissue was obtained by surgery. CT scans repeated monthly during this period demonstrated that the angioma continuously decreased in size. There was no evidence of hemorrhage in the angioma on serial CT scans. The histopathology revealed thrombosis of cavernous vessels with hyaloid changes in the angioma. DISCUSSION: The mechanisms of the decreasing size of the cavernous angioma have often been discussed in relation to spontaneous hemorrhages and resolution. The present case suggests a mechanism in which the spontaneous formation of a thrombus might be the dominant factor for the decrease in size. Thrombus formation may result from low perfusion due to the large size of the angioma.
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3/4. Giant cavernoma of the brain stem: value of delayed MR imaging after contrast injection.

    Cavernous angiomas are vascular malformations composed of slowly perfused, sinusoidal vessels which can be located in any part of the central nervous system. Whereas diagnosis is mostly straightforward in typical cases, some lesions may present in unusual locations or with unusual imaging characteristics. Because of the slow perfusion, contrast enhancement is not regarded as a characteristic imaging feature of cavernomas. We report a large brain stem cavernoma with signs of recent bleeding, in which the differential diagnosis against other mass lesions was facilitated by the demonstration of slow, but intense, contrast enhancement on MRI 1 h after contrast injection. We conclude that contrast enhancement in delayed images may contribute to a safe diagnosis of cavernous haemangiomas and should be performed in atypical cases.
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4/4. Papillary glioneuronal tumor radiologically mimicking a cavernous hemangioma with hemorrhagic onset.

    Papillary glioneuronal tumor is a recently identified low-grade brain neoplasm classified as variant of ganglioglioma. Its salient morphological characteristics are the presence of pseudopapillary structures composed of blood vessels, often hyalinized, lined by uniform small astrocytes and a proliferation of neurocytic cells, eventually admixed with ganglioid and ganglion cells. We present a case of papillary glioneuronal tumor occurring in a 15-year-old female with an unusual hemorrhagic onset. The clinical, morphological and immunohistochemical features are discussed and the published literature is reviewed. This article proposes that papillary glioneuronal tumor should be included in the differential diagnosis of patients with tumoral related brain hemorrhage.
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